Pediatric venous disease is increasing in incidence in both inpatient and outpatient populations. The widespread use of central venous access devices as well as the rising incidence of thromboembolic events in pediatrics is leading to more systemic venous occlusions in both the central and peripheral veins. This review focuses on the etiology, presentation, workup, and general technical considerations of recanalization as well as procedural complications related to pediatric systemic venous occlusive disease. The potential role for pediatric interventional radiology guided treatments will be discussed in detail.
A 14-year-old female with no significant medical history presented with hypertensive urgency, in the setting of 4 to 6 weeks of diarrhea, abdominal pain, headaches, anemia, weight loss, and high blood pressures. Her evaluation revealed signs of a systemic inflammatory process that was most suspicious for inflammatory bowel disease. However, when her hypertension was evaluated with a renal Doppler ultrasound, there were signs of narrowing, stenosis, and hypoplasia that led to a diagnostic angiogram of the abdominal aorta. Full body positron emission tomography scan revealed multiple areas of stenosis and aortic thickening with enhancement compatible with Takayasu arteritis. She received prednisone, methotrexate, and infliximab with marked improvement in her clinical symptoms and inflammatory markers.
Metachromatic leukodystrophy (MLD) is a neurodegenerative disorder caused by the accumulation of lipids called sulfatides throughout the nervous system. Sulfatides can also collect in other organs throughout the body including the gallbladder where they form polyps. Gallbladder polyps rarely have been found to bleed in patients with known MLD, presumably due to polyp shearing. Here we present a case of a child with autism presenting with severe gastrointestinal bleeding and direct hyperbilirubinemia, requiring significant resuscitation and biliary drain placement to tamponade ongoing bleeding. Subsequent neurologic and genetic investigation led to the diagnosis of MLD, with laparoscopic cholecystectomy revealing extensive, elongated gallbladder polyps. Clinicians who care for patients with MLD, including gastroenterologists who manage their progressive oropharyngeal dysphagia, should be aware of the risk for this life-threatening complication. Moreover, pediatric gastroenterologists and hepatologists should maintain a high index of suspicion for MLD in new patients presenting with developmental regression and gastrointestinal bleeding.
Lymphatic malformations are low-flow vascular malformations that are typically apparent in the pediatric population and can cause significant functional limitations and effects on quality of life. While surgical resection has historically been the mainstay of therapy, percutaneous sclerotherapy has garnered increasing popularity due to its efficacy and low complication rates. The role of interventional radiology in the multidisciplinary management of these often complex malformations requires thorough understanding of the disease process. This article will review the pathophysiology, clinical presentation, imaging workup, and management options of lymphatic malformations. Special attention will be devoted to available sclerosants, the mammalian target of rapamycin inhibitor sirolimus, and complex lymphatic anomalies.
Objective
Safe care of central venous access devices (CVAD) requires clinicians be able to identify key CVAD properties from insertion until safe removal. Our objective was to design and evaluate interfaces to improve CVAD documentation quality and information retrieval.
Materials and Methods
We applied user-centered design (UCD) to CVAD property documentation interfaces. We measured expert agreement and front-line clinician accuracy in retrieving key properties in CVADs documented pre- and postimplementation.
Results
The new approach (1) optimized searches for line types, (2) enabled discrete entry of key properties which propagated to the display name, and (3) facilitated error correction by experts. Expert agreement on key CVAD properties improved from 42% to 83% (P < 0.01). Frontline nurses’ perception of key CVAD properties improved from 31% to 86% (P < 0.01). Ease of use scores improved from 15/100 to 80/100 (P < 0.01).
Conclusions
UCD significantly improved data quality and nurse perception of CVAD properties to guide subsequent care.
Thrombogenic superior vena cava syndrome is an uncommon, dangerous complication of long-standing central venous catheter use. The increased use of central venous catheters has resulted in more non-malignant cases of superior vena cava syndrome across all age groups. We present a 5-year-old male with superior vena cava syndrome associated with acute onset of severe upper extremity and facial swelling, dyspnea, and a right subclavian central venous catheter malfunction. The patient was ultimately treated with percutaneous stenting of the superior vena cava with balloon-expandable Palmaz stents following unsuccessful angioplasty, catheter-directed thrombolysis, and percutaneous thrombectomy. This case highlights a relatively uncommon complication in children from long-term central venous catheter access and describes an emerging, minimally-invasive therapeutic alternative that allows for preservation of age-appropriate superior vena cava luminal diameter as patients grow.